POS1457 CLINICAL COURSE AND RISK FACTORS FOR DEVELOPMENT AND PROGRESSION OF INTERSTITIAL LUNG DISEASE IN PRIMARY SJÖGREN’S SYNDROME: A SINGLE-CENTERED, RETROSPECTIVE, OBSERVATIONAL STUDY
Authors: K. A. Lee, B. D. Nam, B. Y. Kim, J. H. Hwang, H. S. Kim
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Interstitial lung disease (ILD) is the most frequent pulmonary manifestation in patients with Primary Sjögren's syndrome (pSS). Data on the clinical and radiographic course based on high resolution computed tomography (HRCT) findings are limited. <h3>Objectives</h3> We aimed to investigate the long-term course and prognostic factors of patients with primary Sjögren syndrome-associated interstitial lung disease (pSS-ILD). <h3>Methods</h3> This single-center, retrospective study included 120 pSS patients who underwent at least two high-resolution computed tomography (HRCT) scans between March 2013 and February 2021. Clinical symptoms, laboratory data, HRCT findings, and pulmonary function test results were collected. Two thoracic radiologists reviewed the HRCT findings. <h3>Results</h3> In patients with pSS without ILD at baseline (n=81), no development of ILD was found on follow-up (median, 2.8 years). In patients with pSS-ILD (n=39), total disease extent, extent of coarse reticulation, and traction bronchiectasis increased on HRCT, whereas the extent of ground glass opacity (GGO) decreased at follow-up (median, 3.2 years) (each <i>p</i>&lt;0.001). In progressive group of pSS-ILD (n=19/39, 48.7%), the extent of coarse reticulation and coarseness score of fibrosis were increased at follow-up (<i>p&lt;</i>0.05). Multivariate logistic regression analysis showed that LDH (OR, 1.012) and diffusing capacity for carbon monoxide (OR, 0.922) were independent risk factors for pSS-ILD at baseline. Usual interstitial pneumonia pattern on CT (OR, 15.237) and follow-up duration (OR, 1.403) were independent risk factors for disease progression in patients with pSS-ILD. In response to glucocorticoid and/or immunosuppressants, GGO decreased, whereas the extent of fibrosis increased even after treatment. <h3>Conclusion</h3> In pSS patients with no ILD during baseline evaluation, no newly developed ILD was identified during follow-up over two years. Progression occurred in approximately half of the pSS-ILD patients with slow gradual deterioration. The extent of fibrosis increased, even after anti-inflammatory treatment. <h3>References</h3> [1]Goh NS, Desai SR, Veeraraghavan S, Hansell DM, Copley SJ, Maher TM et al. Interstitial lung disease in systemic sclerosis: a simple staging system. <i>Am J Respir Crit Care Med</i> 2008;177:1248–54. [2]Hansell DM, Bankier AA, MacMahon H, McLoud TC, Müller NL, Remy J. Fleischner Society: glossary of terms for thoracic imaging. <i>Radiology</i> 2008;246:697–722. [3]Kim YJ, Choe J, Kim HJ, Song JW. Long-term clinical course and outcome in patients with primary Sjögren syndrome-associated interstitial lung disease. <i>Sci Rep</i> 2021;11:12827. Plots are shown for the total extent (A) ground-glass opacities (GGO) (B), fine reticulation (C), coarse reticulations (D), coarseness score of fibrosis (E), and score of traction bronchiectasis (BE). <h3>Acknowledgements</h3> This research was supported by a grant of Patient-Centered Clinical Research Coordinating Center (PACEN) funded by the Ministry of Health &amp; Welfare, Republic of Korea (grant number: HC21C0100). This work was also supported by the Soonchunhyang University Research Fund. <h3>Disclosure of Interests</h3> None Declared.